Macrophage activation syndrome as an initial presentation of systemic lupus erythematosus with sensorineural hearing loss in a young male patient

被引:5
|
作者
Tan, Chou Luan [1 ]
Yahaya, Muhammad Hafizuddin [2 ]
Ahmad, Noor Shahrazat [2 ]
Lim, Chong Hong [1 ]
机构
[1] Hosp Sultanah, Dept Internal Med, Rheumatol Unit, Bahiyah, Alor Setar, Malaysia
[2] Hosp Sultanah Bahiyah, Dept Internal Med, Bahiyah, Alor Setar, Malaysia
关键词
rheumatology; connective tissue disease; systemic lupus erythematosus; haematology (including blood transfusion); pathology; HEMOPHAGOCYTIC SYNDROME; DIAGNOSIS;
D O I
10.1136/bcr-2019-233330
中图分类号
R5 [内科学];
学科分类号
1002 ; 100201 ;
摘要
An 18-year-old male adolescent presented with prolonged high spiking temperature, photosensitive rash, oral ulcers and reduced hearing bilaterally of recent onset. Examination revealed malar rash, vasculitis rash over bilateral palms and soles, oral and buccal ulcers, palpable posterior auricular and inguinal lymph nodes, and reduced hearing bilaterally. His further investigations noted pancytopaenia, elevated transaminases, hyperferritinaemia, low C3 and C4 levels, positive antinuclear antibody, double-stranded DNA and direct Coombs test, while bone marrow aspiration revealed active phagocytic activity suggestive of hemophagocytic syndrome. We made a diagnosis of systemic lupus erythematosus with macrophage activation syndrome. We treated him with pulse intravenous methylprednisolone and his condition improved drastically. Temperature resolved on the next day after intravenous methylprednisolone; bilateral sensorineural hearing loss improved to near-normal hearing after treatment. He remained well during follow-up with a tapering dose of prednisolone.
引用
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页数:3
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